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C850Y

Category 3/4 — Most DruggableConflictingLumenal · predictedσ-1 candidateEditorial
CysteineTyrosine at position 850 · C-terminal lumenal domain (653-869) · WFS1 (Wolframin)

Cysteine → Tyrosine at position 850 in wolframin's C-terminal lumenal domain. ClinVar Conflicting classifications including Wolfram syndrome 1. AlphaMissense 0.975, DynaMut2 ΔΔG -0.42 kcal/mol (destabilising). The C850 partner of C847Y in the inferred C847-C850 disulfide.

Interactive 3D Structure

Wild-type reference
Wild-type C850 — hydrogen bond to C847
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DynaMut2 mutant · C850Y
Mutant Y850 — hydrogen bond to L817 lost (3 contacts lost)
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Bond changes · DynaMut2 interaction analysis

3 lost4 gained2 preserved
Interaction typeWild-type partnerMutant partnerStatus
Hydrogen bondL817Lost
Hydrogen bondC847C847Preserved
Polar contactS816Gained
Polar contactR818Gained
Polar contactC847C847Preserved
Polar contactA852Lost
Van der WaalsC847Gained
HydrophobicR818Gained
HydrophobicC847Lost

Lost / gained / preserved interatomic contacts at the variant residue, from the DynaMut2 (Arpeggio) interaction analysis of the wild-type and energy-minimized mutant structures.

Computational Predictions

DynaMut2 ΔΔG
-0.42kcal/mol
Destabilising — mild
AlphaMissense
0.975
LPath
AlphaFold pLDDT
74
model confidence
Schema
Cat 3/4
Category 3/4 — Most Druggable

Clinical Evidence

ClinVar classificationConflicting classifications of pathogenicity
Review statuscriteria provided, conflicting classifications
Associated conditionsWolfram syndrome 1
InheritanceWolfram syndrome 1 documented.
Population frequency (gnomAD v4)Ultra-rare · AF 0.00025%
cDNA changec.2549G>A
ClinVar accessionVCV000287170
Last evaluated2023/11/07 00:00

Observed at very low frequency in gnomAD.

Structural Context

Position 850 sits in wolframin's C-terminal lumenal domain. The AlphaFold model places C850 within 5 Å of MET851 (2.5 Å), ASN849 (2.5 Å), and CYS847 (3.6 Å — partner of C847Y Atlas card, possible disulfide). The C847-C850 distance of 3.6 Å is the strongest disulfide-distance signal in this batch.

Replacing C850 with tyrosine eliminates this potential disulfide and introduces aromatic volume. Combined with C847Y (Atlas card adjacent), both ends of the inferred C847-C850 disulfide are now known to be pathogenic when mutated.

The |ΔΔG| of 0.42 reflects fold accommodation. AlphaMissense's 0.975 confirms severe functional consequence. pLDDT 74 is borderline but above the IDR threshold.

Amino-acid chemistry
Cysteine (C) → Tyrosine (Y) — thiol-bearing residue replaced by aromatic phenol. Loss of disulfide potential; aromatic introduction.
Position in the protein
C-terminal lumenal domain · position 850 in the ER lumen (pLDDT 74 — borderline).

Druggability Assessment

Category 3/4 — Most Druggable. |ΔΔG| = 0.42 — fold survives. AlphaMissense 0.975 + Wolfram 1 confirm severe functional consequence.

Mechanism is loss of the inferred C847-C850 disulfide. Therapeutic strategy: same C847-C850 microregion as C847Y.

Why this matters

C850Y + C847Y are the two pathogenic variants at opposite ends of the inferred C847-C850 disulfide. The Atlas now contains three identified disulfide-pair targets: C673-C690, C733-C765, C847-C850.
Therapeutic Strategy Handoff · prediction

Feed this card to Wolfram Intelligence

Download the C850Y PDF below and upload it to Wolfram Intelligence to generate therapeutic-strategy proposals — guanidinium mimetics, sigma-1 agonist docking, NAC thiol-capping. NAC is already on the bench-testing list.

Download C850Y PDF card ↓Strategies are AI-generated predictions, not validated therapeutics.

UniProt Domain Annotation

Chain1890 · Wolframin
Topological domain653869 · Lumenal